Sickle cell nephropathy with diffuse proliferative lupus nephritis: a case report
© Kanodia et al; licensee BioMed Central Ltd. 2008
Received: 14 August 2007
Accepted: 28 February 2008
Published: 28 February 2008
Sickle cell nephropathy (SCN) is an important cause of mortality in patients with sickle cell disease. SCA with systemic lupus erythematosus (SLE) is known in children and less common in adults, however diffuse proliferative lupus nephritis (DPLN) with SCN has rarely been reported in adults. It requires early diagnosis and aggressive management.
We present here a 35 years old lady with sickle cell disease who presented with edema, dyspnoea on exertion, pyuria and had raised s. creatinine of 7 mg%. Her biopsy revealed SCN with DPLN. She is on maintenance hemodialysis after 2 months of diagnosis.
DPLN with SCN is a rare entity with poor prognosis, which may be overlooked and needs aggressive management.
Sickle cell nephropathy (SCN) is an important cause of mortality in patients with sickle cell disease (SCD) . SCN includes hematuria, papillary necrosis, urinary concentrating defect, impaired renal acidification and potassium excretion, supranormal proximal tubular function, proteinuria, and renal failure1. Incidence of renal failure in SCD ranges from 5 to 18% . SCN associated with diffuse proliferative lupus nephritis (DPLN) is a rare entity. We present here a 35 year old lady with sickle cell disease (SCD) and DPLN.
A 35 year old female with SCD, presented with pedal and periorbital edema, distension of abdomen, decreased urine output and dyspnoea on exertion since 1 month. On examination, she was pale, with pulse rate of 100/minute, normal temperature, and blood pressure was 160/100 mm Hg. Her abdomen was distended due to moderate ascites however there was no organomegaly/scars. Cardio-respiratory and neurological examination was unremarkable. Ultrasonography showed right kidney, 10.9 × 6.0 cm, left kidney of 11.4 × 5.8 cm, with increased echogenicity and maintained cortico-medullary differentiation. Moderate ascites was present.
On investigations, urine albumin was 500 mg/24 hours, microscopy showed 30–40 pus cells and 3–5 granular casts/high power field, urine culture was sterile, serum creatinine, 7.0 mg%, serum proteins, 4.5 gm/dL; serum albumin, 1.8 gm%, serum bilirubin was 0.5 mg/dL, serum alanine amino transferase was 16 units/L, random blood sugar was 87 mg/dL, serum uric acid was 7.5 mg/dL, serum sodium was 134 meq/L and serum potassium, 5.5 meq/L and positive sickling test was noted at 24 hours. Her hemoglobin was 7.1 gm/dL; total leucocyte count was 7,700/cmm with differential count showing neutrophils, 76%, lymphocytes, 20% and eosinophils and monocytes, each, 2%. Peripheral smear showed few crenated and sickled cells with mild hypochromia and anisopoikylocytosis.
She was treated with Methylprednisolone 500 mg, intravenously for 3 days and Cyclophosphamide, 500 mg intravenously immediately and dose was repeated after 21 days. Then she was switched over to oral Prednisolone, 40 mg/day. Hemodialysis was done on alternate days for 21 days. At follow-up of 2 months, she is on maintenance dialysis with urine output of less than 400 ml/day.
Patients with SCA are known to develop idiopathic nephrotic syndrome with focal and segmental glomerulosclerosis or with membranoproliferative glomerulonephritis. Systemic lupus erythematosus (SLE) has been reported in adults and more frequently in children with SCD [3, 4]. However lupus nephritis with SCN has been rarely documented in adults. It is postulated that a defect in the alternate pathway of complement activity seen in patients with SCD may predispose to immune complex disorders . There are broad spectrum of systemic complications associated with SCD and/or SLE [6, 7]. Mesangial expansion, basement membrane duplication as well as occurrence of immune complex disorders in SCD may delay the unmasking of lesion due to SLE unless serological work-up is done. This is a rare case of DPLN along with SCN in an adult, who has become dialysis dependent.
SCN with DPLN is a rare clinico-pathologic entity which requires timely diagnosis and aggressive management.
Diffuse Proliferative Lupus Nephritis
Sickle Cell Disease
Sickle cell nephropathy
Systemic lupus erythematosus
We are thankful to Mr. Babubhai Patel, Mr. Jaydatt Chudasma and Ms. Pinki Bhavsar for preparing the histopatholgy and immunofluorescence slides of this patient, and Mr. Vipul Gandhi and Mr. Yazdi Wadia for manuscript preparation.
- Stuart MJ, Nagel RL: Sickle cell disease. Lancet. 2004, 364 (9442): 1343-60. 10.1016/S0140-6736(04)17192-4.View ArticlePubMedGoogle Scholar
- Scheinman JI: Sickle cell nephropathy. Pediatric Nephrology. Edited by: Holliday M, Barratt TM, Avner ED. 1994, Baltimore, Williams & Wilkins, 908-919.Google Scholar
- Saxena VR, Mina R, Moallem HJ, Rao SP, Miller ST: Systemic lupus erythematosus in children with sickle cell disease. J Pediatr Hematol Oncol. 2003, 25 (8): 668-71. 10.1097/00043426-200308000-00019.View ArticlePubMedGoogle Scholar
- Flanagan G, Packham DK, Kincaid-Smith P: Sickle cell disease and the kidney. Am J Kidney Dis. 1993, 21 (3): 325-7.View ArticlePubMedGoogle Scholar
- Shariff S, Al-Hemi B, Al Yousif R, Jabreel N, Al Gharreb S, Balaji D: Spectrum of renal disease in sickle cell anemia. Indian J Nephrology. 2006, 16: 157-163.Google Scholar
- Khalidi NA, Ajmani H, Varga J: Coexisting systemic lupus erythematosus and sickle cell disease: a diagnostic and therapeutic challenge. J Clin Rheumatol. 2005, 11 (2): 86-92. 10.1097/01.rhu.0000158549.92844.96.View ArticlePubMedGoogle Scholar
- Saborio Pablo, John J, Scheinman : Sickle cell nephropathy. J Am Soc Nephrol. 1999, 10 (1): 187-92.PubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.